Observational studyNeurology2025
Comparison of the Clinical Spectrum of Juvenile- and Adult-Onset Huntington Disease: A National Cohort and Enroll-HD Observational Study.
Observational study in Neurology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT01574053 (Enroll -HD), which is not on this map. Cited by 6 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Enroll -HD: A Prospective Registry Study in a Global Huntington's Disease Cohort
Who cites it
6 citing papers in PubMed.
- Review
- Early Manifestations, Diagnostic Pathways, and Epilepsy in Juvenile-Onset Huntington Disease: A Three-Patient Case Series and Systematic Review.Brain sciences · 2026Article
- The potential of vitamins in Huntington's disease: bridging mechanistic evidence with translational limitations and future strategies.Metabolic brain disease · 2026Review
- Psychosis in Huntington disease: a short update.Journal of neural transmission (Vienna, Austria : 1996) · 2026Review
- Substantia Nigra as Brainstem Signature in Pediatric-Onset Huntington's Disease: The RAREST-JHD Study.Movement disorders clinical practice · 2026Article
- Observational
Corrections and comments
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Authors and funding
8 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
BACKGROUND AND
objectivesDifferences in clinical characteristics between juvenile-onset Huntington disease (JHD) and adult-onset HD (AHD) are hypothesized but not directly compared. This study compares clinical characteristics occurrence and severity across age-at-onset (AO) subtypes.
methodsUsing the national juvenile-onset HD patient cohort and the international Enroll-HD registry (NCT01574053), we compared childhood-onset JHD (cJHD; AO 0-10), adolescent-onset JHD (aJHD; AO 11-20), and adult-onset HD (AHD; AO 21-65) on proportions of clinical characteristics at onset and psychiatric characteristics in pooled datasets. Kruskal-Wallis test was applied to UHDRS-Total Motor Score (UHDRS-TMS) items of the Enroll-HD dataset to compare the severity of motor disease characteristics 6-10 years after onset.
resultsThe combined datasets provided data from 46 patients with cJHD (mean AO 6.70, 45% female), 243 patients with aJHD (mean AO 16.70, 46% female), and 9,504 patients with AHD (mean AO 44.70, 51% female). At onset, neurocognitive symptoms occurred in 47.50% of patients with cJHD (n = 46; 95% CI 31.80%-63.70%), significantly more often compared with 24.88% of patients with aJHD (n = 209; 19.30%-31.40%) and 15.02% of those with AHD (n = 8,177; 14.30%-15.80%). Psychiatric symptoms occurred in 47.12% of patients with aJHD (95% CI 40.20%-54.10%), significantly more compared with 31.04% of patients with AHD (30.10%-32.00%). Throughout the disease, aggressive behavior occurred in 73.91% of patients with cJHD (n = 46; 95% CI 58.60%-85.20%) and 55.88% of those with aJHD (n = 238; 49.30%-62.30%), significantly more compared with 40.65% of patients with AHD (n = 9,501; 39.70%-41.70%). Psychosis occurred in 23.53% of patients with aJHD (95% CI 18.40%-29.50%), significantly more compared with 12.77% of those with AHD (12.10%-13.50%). The Kruskal-Wallis test revealed significantly higher median UHDRS-TMS scores in one or both JHD subtypes compared with AHD for dysarthria (AHD: n = 4,163, median 1.00, interquartile range (IQR) 0.70; cJHD: n = 12, 2.20, 2.00, DISCUSSION: This study highlights distinct clinical patterns in JHD subtypes compared with AHD. Stratification by age at onset-defined HD subtypes is needed in future studies.
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